Sigmoid colostomy was the first documented surgical procedure for ulcerative colitis. It was not until the 1940s when it became clear that the only definitively curative treatment of chronic ulcerative colitis is total proctocolectomy or, as a compromise, subtotal abdominal colectomy with ileostomy. However, the ileostomy was fraught with technical problems from the outset, including the optimal location of the ostomy site, surgical construction and attachment techniques, and leak proof collection pouches and skin barriers. All of these contributed to a high complication rate and patient dissatisfaction. It was not until the early 1950s when Brooke in the United Kingdom and Crile and Turnbull in the United States proposed that the ileal stoma could be immediately matured into the skin with primary mucocutaneous suturing.46,47 This innovative procedure, coined the “Brooke ileostomy” (Fig. 34-7), when performed after a proctocolectomy, rapidly emerged as the surgical procedure of choice for ulcerative colitis and finally offered patients a curative operation with a reasonably manageable outcome.
Perhaps the earliest attempt to promote a more functional and continent alternative to a permanent ileostomy was proposed by Stanley Aylett of the United Kingdom, who in the early 1950s began performing colectomy and IRA for ulcerative colitis.48 Despite the fact that diseased mucosa remaining in the rectal segment clearly increased the risk of persistent symptoms and cancer of the rectum, he performed nearly 400 such procedures through the mid-1970s, with relatively satisfactory functional outcomes and a low cancer rate.49 The next major advance in the surgical treatment of ulcerative colitis was the description of the continent ileostomy, or Kock pouch by Kock50 in the late 1960s. It was his reasoning that a high-volume, low-pressure reservoir could be constructed and attached to the abdominal wall, which could be emptied using a catheter but otherwise would be continent. To make the reservoir, Kock initially described taking an isolated loop of small bowel that was divided at its antimesenteric border and folded into a U and closed side to side. However, continence was not maintained, and subsequently a valve made out of an intussuscepted segment of small bowel was interposed between the reservoir and the outlet. Patients then passed a tube through the flush stoma to empty the pouch. Patients searching for an alternative to a Brooke ileostomy enthusiastically received the Kock pouch. Although they still had a stoma, they did not have to wear an appliance. Unfortunately, the procedure is technically difficult and despite multiple technical revisions to the procedure, there is a high complication rate and need for reoperation. In addition, the patient still has a stoma, leading surgeons to seek other alternatives. Thus, in the early 1970s, Parks and Nicholls51 and Utsunomiya et al52 independently adopted the concept of the ileal reservoir that Kock had described and anastomosed it to the anal sphincter. Continence and stool frequency was acceptable, and the patients could evacuate via the normal route. In addition, following multiple technical changes, the procedure could be performed with relatively low morbidity, good functional results, improved quality of life, and patient satisfaction. Thus, the IPAA, ileoanal “pull-through” or restorative proctocolectomy, is currently the procedure of choice for most patients requiring surgery.
Currently, there are several options for patients requiring surgery for ulcerative colitis. Subtotal colectomy and ileostomy is often performed in patients requiring surgery urgently or emergently. The more definitive options include colectomy and IRA, total proctocolectomy and end ileostomy or Kock pouch, and IPAA. All have advantages and disadvantages, and the patient must be fully informed about the procedures, including the risks and functional results so that he or she may partake in the decision making. However, irrespective of the procedure, most patients following surgery have an improved quality of life.
Subtotal Colectomy and Ileostomy
Subtotal colectomy and ileostomy is often performed as the first stage prior to a more definitive procedure. The most common indication is in the emergency or urgent situation in patients with acute colitis. Even in patients with severe colitis, including those with severe bleeding, colectomy alone usually results in a dramatic clinical improvement without the morbidity associated with a potentially hazardous pelvic dissection. The inflammatory process may persist in the retained rectum, but it is usually not severe and requires no treatment or perhaps topical medical therapy. Proctocolectomy is only rarely indicated if there is profound hemorrhage from rectal ulceration. A second indication is in individuals with chronic disease who are malnourished and are on high doses of corticosteroids (the equivalent of 30 mg or more of prednisone/day). Finally, if there is uncertainty about whether the diagnosis is ulcerative colitis or Crohn's disease, by removing the colon first, a formal pathologic assessment of the specimen can be made and the rectal disease can be dealt with at a subsequent operation.
While subtotal colectomy is an excellent operation in these situations, it is not a definitive procedure, and unless there is a contraindication, proctectomy should be performed in the future as there is an ongoing risk of malignancy. A cancer may occur without symptoms until it is quite advanced. Surveillance of the rectum in the long term is usually inadequate.
After induction of general anesthesia with endotracheal intubation, patients are placed in the lithotomy position. The bladder should be catheterized. A midline incision provides wide access to the abdomen and does not compromise placement of a stoma on either side of the abdomen. Upon entering the abdomen, an exploratory laparotomy should be performed with particular attention to the terminal ileum to ensure that there is no disease because this would make one think the patient has Crohn's disease. Great care should be taken in the handling of the tissues as they may be extremely friable in severe disease or in cases of prolonged steroid use. This is especially true of the flexures where a walled off perforation may not be apparent. Mobilization usually begins with the right colon. In some patients, the flexures may be easier to mobilize owing to the shortening of the bowel seen in chronic disease. Care must be taken to protect the ureters, duodenum, and spleen. Our preference is to remove the omentum because preserving it may increase the risk of adhesional obstruction. Some surgeons preserve it, arguing that it may limit sepsis from an anastomotic leak in a subsequent IPAA.
The terminal ileum should be divided immediately proximal to the ileocecal valve to preserve bowel length if an IPAA will be performed at a later date. Our preference is to divide the colon in the distal sigmoid with preservation of the inferior mesenteric and superior rectal arteries, as it is easier to mobilize the rectum subsequently and decreases the risk of injury to the left ureter and sympathetic nerves. Perhaps the most important reason, though, is that it permits exteriorization of the rectal stump in patients with severe colitis. Breakdown of the rectal stump occurs infrequently but is the most significant complication following this procedure. In our own series of patients, between 5 and 10% of patients experienced a blown rectal stump depending on the severity of the disease at surgery when the rectum was closed and left within the abdomen.53,54 There is significant morbidity associated with a blown rectal stump, including development of a pelvic abscess or even generalized peritonitis. There are three options for handling the rectum including an exteriorized mucous fistula, closure of the rectum with exteriorization in the subcutaneous tissue, or Hartmann's procedure with placement of a 30F Foley catheter to decompress the stump. The decision about how to handle the rectal stump should be based on the severity of disease and the friability of the rectosigmoid at the time of surgery. An exteriorized mucous fistula is the safest method if the rectosigmoid is very friable but may continually drain a large volume of mucous and blood, necessitating the wearing of a second appliance. Closure of the rectum and its exteriorization in the subcutaneous tissue is often a good compromise in the presence of friable tissue, although outcomes have only been reported by a few centres.53,55,56 In our own experience, one-third of patients with subcutaneous stump closure will go on to develop breakdown of the rectum with persistent drainage and possible wound infections.53 However, serious intra-abdominal septic complications are avoided. The rectal stump may be stapled off in patients where the risk of a leak from the rectal stump is deemed to be small.
There is increasing evidence that laparoscopic subtotal colectomy, even in acute colitis, can be performed safely. Initially, a 12-mm port is inserted at the umbilicus, with ports then inserted in each of the four quadrants lateral to the rectus sheath. Mobilization of the colon from its peritoneal attachments is performed in much the same fashion as in the open approach. Retraction of the small bowel is facilitated by tilting of the operating table. Division of the mesocolic vessels is made close to the bowel wall and can be achieved intracorporeally with use of clips, vascular staplers, or an energy device. Mobilization of the transverse colon and division of the middle colic vessels may be difficult and tedious. Thus, the colon can be delivered through the umbilical port site, and division of the mesentery of the transverse colon can be completed extracorporeally. The terminal ileum and distal sigmoid colon can be divided using stapling devices and extracted through the umbilical site. Alternatively, the specimen can be extracted through a Pfannenstiel incision, through which a subsequent proctectomy can be performed or through the ileostomy site that may provide some additional cosmetic benefit.
Early concerns about the safety of the laparoscopic approach, particularly in cases of acute severe colitis, limited its introduction. Evidence is now emerging from specialist centers that laparoscopic subtotal colectomy is not only safe but may confer potential benefits in terms of postoperative recovery. However, many studies are limited by the inclusion of patients with noncolitic indications for colectomy. Several series have reported a reduced length of stay and time to bowel function, at the expense of an increase in operating time with the laparoscopic approach.54,57–59 A few comparative observational studies have even suggested that time to restorative proctocolectomy may be reduced by employing the laparoscopic approach to initial subtotal colectomy.60,61 There are currently no randomized controlled trials comparing outcomes between the laparoscopic and open approaches, so the benefits reported in these studies should be viewed with caution in light of their inherent selection bias. Data on the late complications of laparoscopic subtotal colectomy, such as incisional herniation and small bowel obstruction, are lacking, which may reflect the fact that many of these patients go on to subsequent proctectomy within a few months of surgery.
Colectomy and Ileorectal Anastomosis
Because ulcerative colitis almost always involves the rectum, there are limited indications for colectomy and ileorectal anastomosis (IRA). If there is sparing of the rectum, a colectomy and IRA may be preferred over IPAA because of the concern that the patient might have Crohn's rather than ulcerative colitis. However, this is an infrequent occurrence.
Patients may continue to suffer from symptoms of proctitis following surgery. They also continue to be at risk of developing dysplasia and cancer, so they require ongoing surveillance. On the other hand, the advantages to an IRA are that it avoids the risk of pelvic nerve injury and poor perineal wound healing. Generally, the procedure can be performed in one stage.
The extirpative phase of the operation is similar to that of colectomy and ileostomy. The superior hemorrhoidal vessels may be preserved if the anastomosis is performed in the distal sigmoid. More often, the anastomosis is performed at the level of the sacral promontory, and the superior hemorrhoidal vessels are divided. Our preference is to perform a hand-sewn anastomosis because usually the rectal wall is inflamed and the risk of a leak may be lower when a hand-sewn anastomosis is performed rather than a stapled anastomosis. The anastomosis may be performed either as an end-to-end or end-to-side anastomosis. Some suggest defunctioning the anastomosis with a loop ileostomy, but, in this era, if the anastomosis is that tenuous, another surgical option should be considered.
Proctocolectomy and Ileostomy
The intestinal component of chronic ulcerative colitis is cured once the colon and rectum are removed. Therefore, total proctocolectomy with the Brooke ileostomy has historically been the operation of choice. The advantages of this procedure are the following: It can often be performed as a one-stage procedure, the disease is eliminated so patients no longer require medical therapy, and there is no longer the risk of developing a malignancy. The disadvantage is that individuals have a permanent ileostomy. Patients having surgery for ulcerative colitis tend to be young, physically active, and single, so there may be social implications associated with a permanent stoma. While patients feel quite negatively about the prospect of having a stoma preoperatively, studies have consistently shown that most patients are accepting of an ileostomy and that their quality of life is very high postoperatively.62–64 Only a very small proportion of patients have significant psychological problems dealing with the stoma.
In the modern era, total proctocolectomy is typically performed in older patients, in those with significant comorbidities or in those who are not candidates for IPAA. The latter includes patients with low rectal cancers, and those who have perianal disease, have had a prior anorectal surgery or a small bowel resection.
Total proctocolectomy can be performed either as a single-stage procedure, or in the two stages: subtotal colectomy followed by abdominoperineal resection of the rectum.
Patients are placed in the lithotomy position with the buttocks over the edge of the table. The buttocks should be taped apart and the anus sewn shut with a silk purse-string suture. A 1 L of saline bag may be placed under the sacrum to aid with exposure. The perineum should be widely prepped and draped. The vagina should be prepped to allow the surgeon to insert a finger into the vagina to guide the perineal dissection. Care should be taken to protect the peroneal nerves and avoid compression of the posterior compartments of the lower leg. Compartment syndrome after an extended period of time in lithotomy has been described. The arms should be tucked to the patient's sides and padded to protect the hands and forearms. A urinary catheter is mandatory to decompress the bladder. The use of a nasogastric tube is usually not necessary. The abdomen should be entered through a long midline incision, and the colectomy is performed as described previously. In this case, the inferior mesenteric vessels should be divided. A low ligation is preferred to minimize the likelihood of sympathetic nerve injury, unless dysplasia or a cancer was identified preoperatively.
The rectum is mobilized posteriorly in the plane between the fascia propria of the rectum and the presacral fascia posteriorly similar to that for a total mesorectal excision for rectal cancer. The plane is entered after ligating the superior hemorrhoidal/inferior mesenteric vessels. Care is taken to identify and protect the left ureter and the sympathetic nerves. The serosa covering the rectum is scored anteriorly and laterally on both sides. The dissection to the pelvic floor is carried out posteriorly first, extending laterally and then anteriorly. The dissection below the peritoneal reflection differs from that of a cancer operation in that the rectum is skeletonized of its mesorectum in order to reduce the risk of parasympathetic nerve injury. Anteriorly, the dissection is performed on the rectal side of Denonvilliers' fascia to avoid injury to the vagina or the seminal vesicles and prostate. Massive hemorrhage due to injury to the presacral or internal iliac vessels can occur during this part of the dissection, but it should be a rare event as compared to when a more radical oncologic procedure is performed. Bleeding occurs due to misadventure when the wrong planes are entered posteriorly or laterally. Control of bleeding from the presacral veins can be particularly problematic as these vessels can retract into the sacrum and are not easily clamped or tied off. A suture ligature through the periosteum of the sacrum or a sterile tack nailed into the sacrum may be required. On rare occasions, tight packing of the pelvis with sponges may be required with removal of packs 24 to 48 hours later. Similarly, injuries to the vagina in women, or the urethra in men, are possible complications during pelvic and perineal dissection, but they occur rarely and are due to dissection in the wrong plane. Vaginal injuries can be repaired primarily whereas urethral injuries are best managed by a urologist.
Once the abdominal dissection is completed to the level of the levator muscles, the perineal dissection is performed. An intersphincteric dissection is preferred to a wide resection as performed in patients with rectal cancer unless a low-lying cancer is identified preoperatively. In so doing, the external sphincter and levator muscles are preserved. This minimizes the size of the perineal defect and decreases bleeding and wound complications. A solution of dilute epinephrine is injected into the intersphincteric plane to decrease bleeding and make the intersphincteric plane more obvious. The dissection begins posteriorly until the perineal and abdominal operators hand-touch, whereupon, guided by the abdominal operator with a hand in the pelvis posterior to the rectum, the perineal surgeon enters the pelvis just anterior to the tip of the coccyx with a pair of curved Mayo scissors. The perineal surgeon may then hook the levator muscles with a finger laterally in either direction and divide the muscles with electrocautery. Once the posterior and lateral dissection has been completed, the rectum can be brought out of the perineum to facilitate anterior dissection. Alternatively, the abdominal surgeon may direct the perineal surgeon as he or she passes scissors anteriorly between the rectum and the vagina or prostate into the abdominal cavity. Then the levator muscles may be divided laterally to complete the dissection. Once hemostasis is achieved, the pelvis is irrigated through the abdominal wound and drained through the perineal wound. A drain is placed in the pelvis through a separate incision in the abdominal wall to prevent accumulation of blood or serous fluid that may subsequently drain through the perineum. The skin may be closed or the perineum may be closed in layers, approximating the pelvic floor muscles and skin. Absorbable sutures are typically used in the skin.
An ileostomy is constructed in the right lower quadrant that has been marked preoperatively. In order to facilitate its construction, a high ligation of the ileocolic vessels may be performed. There is usually an avascular window approximately 15 cm in length between the ileocolic vessels and the superior mesenteric vessels. The mesentery of this segment of bowel can be narrowed so the bowel is supplied by a marginal artery running along its edge. The ileostomy should be brought out through the aperture so it is not under tension. The fascia and skin of the abdominal wall should then be closed prior to maturation of the ileostomy. Also, before doing so, the mesentery can be sutured to the posterior abdominal wall to prevent an internal hernia. The ileostomy should be matured so it has a height of approximately 2 cm and it sits upright (see Fig. 43-7).
Following total proctocolectomy, a Kock pouch or continent ileostomy may be constructed instead of a conventional ileostomy. The advantage of this operation is that it is a curative procedure that potentially offers improved quality of life because patients are continent and do not have to wear an appliance. The main disadvantage is that the procedure is technically challenging and therefore the complication rate is high. Most of the complications are valve related, and, if they do occur, patients generally require reoperation. Reported long-term failure rates are in the order of 10–40%.65–68 The second disadvantage is that even though patients are continent, they still do not evacuate via the normal route and must insert a tube to empty the pouch. For both of these reasons, the Kock pouch has been rarely performed since the introduction of IPAA. The main indication now is in patients who have already undergone a total proctocolectomy and ileostomy and thus are not candidates for an IPAA. Also, individuals who have had a failed IPAA or who have anal disease may be candidates.
The Kock pouch can be constructed following extirpation of the colon and rectum as described previously or, alternatively, it can be constructed at a later date with takedown of the conventional ileostomy.
The reservoir is made from two or three limbs of small bowel. Although the initial description was that of a two-limb pouch, a three-limb pouch is usually performed now. Approximately 55 cm of terminal ileum is used to construct the continent ileostomy: a 5- to 10-cm segment for the outlet, 15-cm for the nipple valve, and 30-cm for the reservoir itself. The reservoir is constructed by suturing the three 10-cm limbs of small bowel to form the posterior wall of the reservoir. Then the 15-cm segment of small bowel is intussuscepted to form the nipple valve. The valve is maintained by stapling the intussuscepted segment with three firings of the GIA 80-mm stapler, two of those firings being on either side of the mesentery. Then the anterior wall of the reservoir is suture-closed. Because of the problems of slippage of the valve, the valve can be stabilized with several maneuvers, including a sling of fascia or soft mesh through the mesentery and around the fundus of the pouch.69–71 This anchors the pouch to the posterior wall of the abdomen and provides support for the mesentery of the small bowel that forms the nipple valve. Other maneuvers include defatting the mesentery or stapling the valve to the wall of the pouch.71 All of these have been used by proponents, but unfortunately valve slippage continues to be a significant complication. Of all the maneuvers, insertion of a mesh is most effective, but it is at the expense of fistula formation from erosion of the mesh into the fundus of the pouch.
Once the pouch is completed, the outlet can be brought up through an aperture low in the abdomen and sutured to the fascia of the anterior abdominal wall. Then the stoma is matured flush with the skin. A catheter is inserted to ensure that it can be passed easily. It is usually left in situ for 2–3 weeks until the pouch has become fully adherent to the abdominal wall (Fig. 34-8).
The stoma of a Kock pouch is made flush with the skin. A tube is inserted into the pouch to empty it.
Ileal-Pouch Anal Anastomosis
Ileal-pouch anal anastomosis (IPAA) is the procedure of choice for most patients requiring surgery for ulcerative colitis. Its major advantage over other procedures is that the normal route of evacuation is maintained and a permanent ileostomy is avoided. The reported outcome is also satisfactory in patients who have indeterminate colitis, but Crohn's disease is generally considered a contraindication.72,73 Regimbeau and colleagues reported a low failure rate of 10% in a series of 41 patients who had an IPAA constructed for Crohn's disease, but most other series report failure rates up to 50%.74 Furthermore, there do not appear to be any factors that predict which patients will have a good outcome.72
Because IPAA can now be performed safely with relatively few complications, there are fewer relative contraindications. IPAA may be performed in older patients although functional results tend to worsen with age.75 Thus, individuals older than 60 years should be fully informed of all options, particularly total proctocolectomy and ileostomy. While most patients dread the thought of having an ileostomy when they are considering surgical options, in reality quality of life tends to be excellent with an ileostomy and most patients adjust well to it. A decision whether to perform an IPAA should be based on the age and comorbidities of the patient as well as the status of the anal sphincter. Perianal disease is usually a contraindication because of the concern that the patient may have Crohn's disease, as well as concerns about healing. However, IPAA may be considered in selected patients provided the anal disease can be eradicated prior to performing IPAA without compromising the sphincter.76
Patients with cancer may also be considered for IPAA provided the oncologic operation is not compromised. Thus, while IPAA may be a satisfactory option for patients with colon cancer, patients with low rectal cancers or those requiring neoadjuvant or postoperative radiation generally are not candidates. Furthermore, patients who do not have a confirmed cancer in the rectum but have high-grade dysplasia also should not have IPAA because of the high probability that cancer is in fact present and it may be inadequately excised. Although there are a limited number of reports, most patients who have a pouch and have had pre- or postoperative radiation have poor functional results and often lose the pouch. Last, patients with advanced disease should probably undergo one of the other less complicated surgical options such as subtotal colectomy or total proctocolectomy. Depending on the site of the cancer, colectomy may be a good alternative because it can be performed with low morbidity and in the future an IPAA could be considered if the patient survives.
Various modifications to IPAA have been described since it was initially introduced and there still is some variability in how the procedure is performed. First, the procedure can be performed in one, two, or three stages. A one stage operation (removal of the colon and rectum and construction of the pouch without a defunctioning ileostomy) is generally not performed because the reported ileoanal anastomotic leak rate is high. However, several authors have reported acceptable results with this surgical approach.77–79 Generally, though, a one-stage procedure should not be performed by the inexperienced surgeon or in patients who are in suboptimal condition.
Two-stage procedures can be performed as colectomy, IPAA construction and a defunctioning ileostomy initially followed by closure of the ileostomy at a later date. Alternatively, a subtotal colectomy can be performed first, and at a subsequent procedure a proctectomy can be performed with construction of a pouch. Then, the defunctioning ileostomy can be omitted (two-stage procedure) or can be performed (three-stage procedure).
Our preference is to perform a two-stage procedure. In patients who require surgery urgently for acute colitis or are in suboptimal condition caused by poor nutritional status or are on more than the equivalent of 30 mg of prednisone per day, a subtotal colectomy is usually performed. At a second operation we would plan to do a proctectomy and construct the pouch without constructing an ileostomy unless there were intraoperative complications, the pelvic dissection was particularly difficult with significant blood loss, there was tension on the ileoanal anastomosis (IAA), or the IAA was incomplete. Patients who have surgery electively usually have a two-stage procedure also, but this includes proctocolectomy, pouch construction, and a defunctioning ileostomy. After 3–4 months, the ileostomy is closed after a Hypaque study has shown that the IAA and pouch are intact.
Several pouch configurations have been described, including J-, S-, and W-shaped pouches.80 The J-pouch is the preferred type as it is technically easier to create and can be fashioned using a linear stapler. W-pouches are advocated because of their increased reservoir capacity, but this effect is seen only initially; long-term studies have shown no difference in bowel function between pouch types. S-pouches tend to be tedious to construct as they must be hand-sewn, but they are preferred when a hand-sewn IAA is required as they provide more length to reach the anal canal, the pouch fits through the canal easier, and the configuration allows for an end-to-end IAA.
The last area of controversy is whether the IAA should be hand-sewn or stapled.81,82 Although mucosectomy and hand-sewn anastomosis was the technique described in the first descriptions, stapled anastomosis is now the preferred method. It is technically easier and quicker. Performing a hand-sewn IAA is usually more difficult, especially in obese individuals, because the pouch may be under tension. This is rarely the case if the anastomosis is stapled. Functional results also tend to be better with a stapled IAA. On the other hand, a few centimeters of rectal mucosa remain if the anastomosis is stapled and thus the disease is not eliminated. Concern has been raised over the risk of ongoing inflammation or cancer developing in the rectal remnant. In fact, even if a mucosectomy is performed, the disease is not completely eradicated and there have been a few reported cases of cancer arising after mucosectomy.83 Ongoing inflammation requiring treatment is a rare occurrence.84 Thus, it is our preference to perform a stapled IAA unless the patient has a cancer or dysplasia elsewhere in the colon and, if so, then perform a mucosectomy and hand-sewn IAA if it is technically possible.
Surgical Technique—Open Ipaa
IPAA surgery can be divided into two phases: extirpative and reconstructive. The colonic and rectal dissection is performed similar to the abdominal dissection performed for total proctocolectomy. If a stapled IAA is planned, a 30-mm transverse linear cutting stapler can be used to staple off the rectum at the level of the levator muscles. The intent is to leave 1–2 cm of rectal mucosa. If a hand-sewn anastomosis is planned, the rectum can be divided at this level.
Some surgeons preserve the ileocolic vessels and divide one of the arcades to increase the length of the mesentery, but this is not necessary in our experience.85 Rather, it is our preference to divide the ileocolic vessels close to their takeoff from the superior mesenteric vessels. That will add a few centimeters of length to bring the pouch down to form the IAA. The terminal ileum is divided with a linear stapler just proximal to the ileocecal valve.
The reconstructive phase of IPAA begins with full mobilization of the small bowel. If the patient had a subtotal colectomy previously, this begins with takedown of the patient's end ileostomy. The distal 1–2 cm of the ileum is resected with a linear stapler. At our center, this staple line is oversewn with 3-0 absorbable suture in a Lembert fashion, as leaks from the staple line have occurred. All adhesions between loops of small bowel are divided, and the mesentery of the small bowel is mobilized up to the level of the duodenum. If the IAA is stapled, “length” is usually not a concern, but, if a mucosectomy and hand-sewn anastomosis is performed, the small bowel mesentery must be fully mobilized back to the duodenum. Some authors have recommended scoring the peritoneum, but, if this is done, there is a risk of tearing the mesentery and vessels if there is tension on the mesentery when the small bowel is brought down to the pelvis to construct the IAA.
Next, the ileal pouch is fashioned (Fig. 34-9). Before doing so, the bowel should be assessed to see if it will reach the anus. Generally, if the small bowel mesentery in the line of the superior mesenteric vessels stretches beyond the pubic symphysis, it should be adequate to perform a stapled IAA. To construct a J-pouch, the terminal ileum is folded over on itself creating two limbs approximately 15 cm long. Our preference is to orient the pouch so the mesentery lies on the right side somewhat anteriorly and the pouch lies in the hollow of the sacrum with the afferent limb of small bowel entering the pouch on the left side of the pouch. An enterotomy is made on the antimesenteric aspect of the apex of this fold, and two passes of an 80-mm linear stapler are used to create the pouch. A 2-0 polypropylene purse-string suture is placed at the enterotomy to secure the endoanal stapler anvil in place, and a double-staple IAA is constructed using a 28-mm circular stapler. The anvil in the pouch is attached, and the stapler is closed with care not to incorporate adjacent tissue, such as the vagina in women, into the staple line. The orientation of the pouch is confirmed, and the stapler is fired and removed. The proximal and distal tissue donuts are inspected for size and continuity. The pouch is then tested for a leak by advancing a 30F rectal tube into the pouch through the anus. A rectal tube is inserted and secured in place if no ileostomy is constructed and the abdomen is then closed. Otherwise, an ileostomy is brought up through an aperture in the right lower quadrant. Typically the ileostomy is constructed approximately 30 cm proximal to the pouch but varies depending on several factors, the most significant being the weight of the patient.
A. S pouch is constructed from 3 limbs of small bowel each approximately 10 cm in length. The outlet should be approximately 1–2 cm in length. B. J pouch is constructed with 2 limbs of terminal ileum each approximately 15 cm in length. C. An enterotomy is made at the apex of the J pouch. D. Two passes of an 8 cm linear stapler is passed through the apex of the J pouch to construct the pouch.
If a hand-sewn anastomosis is planned preoperatively, the buttocks are taped apart to facilitate access. A Lone Star retractor (CooperSurgical, Inc., Trumbull, CT) is placed at the dentate line to draw the rectal mucosa into the anal canal. A solution of dilute epinephrine is injected submucosally, and the mucosectomy is performed with Metzenbaum scissors or electrocautery starting at the top of the dentate line. Our preference in this situation is to construct an S-shaped pouch that is performed by leaving a 1- to 2-cm outlet and then suturing three limbs of approximately 10 cm of small bowel together. A Babcock clamp is inserted through the anus to grasp the pouch, and the pouch outlet is brought through the anal canal to the anus. This can be difficult, and having two experienced surgeons, one guiding the pouch from above and the second one as the perineal operator, is advantageous during this short but critical part of the operation. Once the pouch is positioned in the pelvis, the stapled end of the S-pouch can be excised or an enterotomy at the apex of the J-pouch can be made and a hand-sewn anastomosis between the ileal pouch and the dentate line can be completed using interrupted 2-0 absorbable sutures beginning with sutures placed in the anterior, posterior, and either lateral position, and then circumferentially until the anastomosis is complete.
Surgical Technique—Laparoscopic Ipaa
Evidence regarding outcome following laparoscopic IPAA is more widespread than for subtotal colectomy. Laparoscopic IPAA appears to confer similar short-term advantages as laparoscopic subtotal colectomy, with a reduction in length of stay, blood loss, and return of bowel function being reported by several large case-matched series.86,87 Only one randomized controlled trial has been published to date and this showed that laparoscopic proctocolectomy and ileal anal-pouch anastomosis took 77 minutes longer.88 No differences in length of stay, morbidity, or long-term functional outcome were demonstrated in this trial, although higher body image and cosmesis scores were reported in the laparoscopic arm.89 Laparoscopic surgery has been associated with a reduced tendency to adhesion formation,90 but there are insufficient long-term follow-up data to determine whether there will be a subsequent reduction in the incidence of small bowel obstruction in patients undergoing restorative proctocolectomy. The risk of infertility in women may be decreased with the laparoscopic approach. A marked increase in operating time appears to be a universal finding as surgeons seek to overcome their learning curve with this procedure.
The colectomy is performed as described previously. The vascular supply of the colon is best divided intracorporeally. Similarly, the rectal dissection is performed in the same fashion as the open approach with dissection in the mesorectal plane above the peritoneal reflection and close to the rectal wall below it, continuing to the level of the levators. The rectum is divided at the pelvic floor, 1–2 cm above the dentate line, with a 30-mm stapler.
Our preference is to make a Pfannenstiel incision for the extraction site. If necessary, dissection of the lower rectum can also be completed through this incision. Also, it allows for better positioning of the transverse linear stapler on the rectum. The pouch is fashioned extracorporeally, and the IAA is constructed using the double-stapled technique. Care must be taken to ensure that the mesentery of the pouch is oriented correctly without any twisting. Indications for defunctioning the IPIAA are the same as for the open procedure.
The technical complexity of laparoscopic subtotal colectomy and IPAA has renewed focus on the potential benefits of the hand-assisted technique. Recent studies have demonstrated that the hand-assisted technique significantly reduces operating times in restorative proctocolectomy without compromising short-term outcomes.91,92 A longer incision is usually required to permit manual access, and this has raised concerns about subjecting the abdominal viscera to excessive trauma.93 It is not clear whether the hand-assisted technique actually maintains the true minimally invasive characteristics of straight laparoscopic surgery, as it may provoke a more dramatic inflammatory response. Data are lacking on the long-term outcomes between the two techniques, although early reports suggest that there is no difference in the rate of incisional herniation and small bowel obstruction.94 A more selective approach to the use of the hand-assisted or straight laparoscopic technique may eventually develop. For example, a troublesome colonic mobilization may be facilitated by the use of a hand port, whereas a more straightforward case could be continued using the straight laparoscopic technique.
Intra-abdominal adhesions develop in virtually all patients undergoing major abdominal and pelvic procedures. Although adhesions do have beneficial effects, they are also the primary cause of small bowel obstruction after abdominal surgery. Patients who have a total extirpation of the colon and rectum are at particularly high risk for the development of small bowel obstruction, possibly because of the combined abdominal and pelvic dissection, and sometimes need for multiple operations.
In patients having IPAA, the reported cumulative risk of small bowel obstruction ranges from 12 to 35% with follow-ups of 2.5–68 months.95 The risk of small bowel obstruction was 8.7% at 30 days, 18.1% at 1 year, 26.7% at 5 years, and 31.4% at 10 years in our own series. However, most patients did not require reoperation. The reoperative rate was 2.7% at 1 year, 6.7% at 5 years, and 7.5% at 10 years. For patients requiring surgery, the most common sites of the obstruction were pelvic adhesions in approximately a third and the ileostomy closure site in 21%. The risk factors for developing a late small bowel obstruction were a previous diverting ileostomy and pouch reconstruction.
Because of the importance of the problem, various strategies have been tried. Beck and colleagues reported on 183 patients who had a barrier substance (Seprafilm) inserted at the time of construction of the pouch.96 A significantly smaller proportion of patients in the Seprafilm group had adhesions to the abdominal wall (49 vs 94%). However, the rate of septic complication rate was significantly higher (13.5 vs 5.1%, p < .001) when Seprafilm was placed around the anastomosis.96 Subsequently, in a larger study that included over 1700 patients, these authors reported that the rate of small bowel obstruction was not decreased as a result of Seprafilm use, although significantly fewer patients required surgery for small bowel obstruction over a 5-year follow-up period (1.8 vs 3.4%, p < .05).97
A laparoscopic approach to surgery is thought to result in decreased adhesion formation and a lower risk of subsequent small bowel obstruction. Indar et al reported on a series of patients who had undergone laparoscopic IPAA and found that the majority (68%) had no adhesions to the abdominal wall and 71% of women had no adnexal adhesions.98 The long-term effect on the incidence of small bowel obstructions remains to be demonstrated.
Complications related to the ileostomy occur frequently. A wide range of complications may occur and they may occur immediately after surgery as well as later in follow-up (Table 34-3). Many can be dealt with nonoperatively. An experienced enterostomal nurse is essential to provide education to the patient and handle skin problems, including those related to irritation, allergies, and yeast infections. In addition, he or she can advise on appliance-related problems. A well-constructed stoma is essential as quality of life is directly correlated with the function of the stoma.99
Table 34-3: Ileostomy Complications ||Download (.pdf)
Table 34-3: Ileostomy Complications
|Early Complications||Late Complications|
|Ischemia/necrosis||Parastomal ulcers and abscesses|
|Mucocutaneous separation||High ileostomy outputs|
Complications are frequent occurrences in patients with loop ileostomies performed to defunction IAA. Approximately one-third of patients will experience high ileostomy outputs. In most instances, this complication can be managed with dietary modifications and antidiarrheal agents such as loperamide and/or codeine. If the outputs remain high, intravenous fluid supplements may have to be given on a regular basis until the ileostomy is closed. Feinberg and colleagues reported that approximately 20% of patients required hospital admission for dehydration.100 Occasionally the ileostomy has to be closed early.
Closure of the loop ileostomy is also associated with a relatively high risk of complications, the most significant of which is an anastomotic leak.100–102 Delaying closure for several months may decrease the difficulty of mobilizing the bowel and decrease the risk of complications. The high complication rate related to the ileostomy has led some surgeons to question the need for an ileostomy. However, the morbidity of a loop ileostomy must be balanced against the potential morbidity of a leak if the pouch is not protected.
Patients who have permanent ileostomies may suffer from problems such as retraction, prolapse, and parastomal hernia. Approximately 10–20% of patients will require revision of their stoma after ten to 20 years of follow-up. Parastomal hernia is caused by enlargement of the stomal aperture. It is seen more frequently in obese patients. If the patient is asymptomatic, no treatment is required. If, however, the patient has problems with retraction of the stoma, difficulty maintaining an appliance, or recurrent small bowel obstructions, surgery may be required. A number of options are available. Local repair of the hernia performed by tightening the fascia is usually unsuccessful. Thus, the recommended treatment has been resiting of the stoma to another location. However, more recently various repairs, performed either open or laparoscopically, have been described where mesh is inserted to repair the defect. Promising results have been reported although most series are small and have short follow-up. It is a difficult problem because the risk factors that led to the hernia initially are usually still present and therefore the recurrence rate is high.
Peristomal ulcers and fistulas occur more commonly in Crohn's disease than in ulcerative colitis. Often, it is a signal that the patient has recurrent disease in the prestomal small bowel. Thus, if a fistula occurs in a patient who is presumed to have ulcerative colitis, one should be suspicious that the diagnosis is incorrect and the small bowel should be examined for signs of Crohn's disease. On the other hand, pyoderma gangrenosa is seen more frequently in patients with ulcerative colitis (Fig. 34-10). Because pyoderma is related to disease activity, it may occur following subtotal colectomy in patients whose rectum has been left in situ. If so, proctectomy should be performed as well as resiting of the ileostomy.
Pyoderma gangrenosum affecting the peristomal region.
Minor wound infections, defined as wound dehiscence of less than two cm in length, stitch abscesses, or sinus tracts have been found to occur in as many as 45% of IBD patients undergoing resection.103 Major complications, including wound failures of more than 2 cm in length, perineal abscesses, or any wound complication requiring readmission or reoperation, occur in as many as 8% of IBD patients undergoing resection. Perineal wound infections and/or dehiscence leads to prolonged and/or delayed healing of the wound, and, in some cases, nonhealing.
Approximately 10–20% patients may have chronic perineal sinuses, defined as a perineal wound that fails to heal by 6 months following surgery. In many cases, it may be relatively asymptomatic and then nothing may need to be done. Management of symptomatic chronic perineal sinuses can be extremely challenging and may require repeated debridement under anesthesia with multiple dressing changes, including the use of vacuum-assisted closure techniques and even flap closure for larger, more complicated wounds.104,105
Genitourinary dysfunction is not an infrequent complication of pelvic surgery. The cause is probably multifactorial. It is likely due to both physical and psychological factors. Injury to the sympathetic and parasympathetic nerves may occur during the pelvic dissection. Several maneuvers should be performed to minimize the risk, including ligation of the inferior mesenteric vessels beyond the takeoff of the left colic vessels, ensuring that the dissection is in the plane of the fascia propria of the rectum at the sacral promontory and close dissection of the mesorectum at and below the level of the peritoneal reflection. Retrograde ejaculation is due to injury to the sympathetic nerves whereas impotence is a result of injury to the parasympathetic nerves. Both may occur temporarily or permanently and partially or totally. Referral to a urologist with appropriate urodynamic testing is indicated if dysfunction persists. The reported rates for impotence and retrograde ejaculation are less than 5%. However, most studies do not use validated instruments to assess sexual dysfunction. Davies et al reported on a cohort of 59 male patients who were assessed prospectively before and again after having IPAA using a validated instrument.106 They found that male sexual function and erectile function scores remained high 12 months following surgery (mean International Index of Erective Function score 51.7 preoperatively versus 58.3 at 12 months postoperatively). Furthermore, the prevalence of abnormal sexual function decreased from 33.3% before to 22.7% after surgery.
It is only recently female sexual function following pelvic surgery has been assessed. Bambrick and colleagues reported that women reported a significant increase in vaginal dryness, dyspareunia, pain interfering with sexual pleasure and limitation of sexual activity because of concerns of stool leakage following IPAA.107 On the other hand, there was no significant change in sexual desire, frequency of sexual intercourse or satisfaction of sexual relationship. The findings are interesting although, due to the sensitive nature of the questions, the response rate was only 35% so the results may be biased. In a recent prospective study by Davies et al, approximately 70% of female patients were found to have scores indicating sexual dysfunction preoperatively using a validated instrument, the IIEF.106 Postoperatively, there was a significant improvement with only 26% reporting sexual dysfunction 6 months following surgery. Improved overall physical well-being after surgery has been suggested as the reason for the improvement.
It is becoming more evident that female fertility is reduced following surgery for ulcerative colitis although it varies with the operative procedure. Oresland and colleagues performed hysterosalpingograms on 20 women who had had a total proctocolectomy and found occlusion of one or both of the tubes in 18.3%.108 Olsen and colleagues reported on 343 female patients followed 10–40 years following surgery for ulcerative colitis and compared them to a reference population of 1200 women in Denmark.109 Surgery significantly reduced the ratio of patient to reference population fecundability (ability to conceive) to 0.20 whereas there was no significant difference in the rates following diagnosis of ulcerative colitis until the time to surgery. They also reported that 29% of women who did conceive required in vitro fertilization. Johnson and colleagues reported similar findings.110 In a cohort of 147 female patients who had IPAA with a mean follow-up of 7.2 years, 38.1% reported problems with fertility compared to a Canadian national average of 8%. Furthermore, 96% of women who wished to become pregnant preoperatively were successful compared to only 55% postoperatively.
The impairment is likely due to adhesions caused by pelvic surgery. This is supported by the results of another study by Olsen and colleagues that showed that fecundity was not decreased in women having an IRA for familial polyposis.111 A study in female patients suffering from ulcerative colitis showed similar results.112 Colectomy and IRA likely has a minimal effect on fertility in women having surgery for ulcerative colitis because there is no pelvic dissection whereas following IPAA the tubes and ovaries are often buried behind or adherent to the pouch.
To date, there are no maneuvers that have been shown to be effective in reducing the risk of infertility. One simple maneuver is to ensure that the ovaries lie above the pouch and perhaps are tacked in this position. Application of barrier agents, although unproven, may be worthwhile. A laparoscopic approach has been shown to decrease adhesions within the abdomen, but less is known about scarring in the pelvis and whether there is less effect on fertility.
Many women who have had IPAA have not started or completed their family. Studies have reported that approximately 45% of women attempted to become pregnant following surgery, but only 56% were successful following IPAA.110 Thus, women must be counseled regarding this risk preoperatively. Although infertility may be increased following total proctocolectomy or IPAA, deferring surgery until a woman has completed her family is unlikely to be a feasible option. Women who are referred for surgery typically have active disease that has become refractory to medical management. For women with active disease who require surgery, one consideration is to perform a colectomy with end ileostomy and defer IPAA because previous studies have shown that colectomy alone does not decrease fertility. While this may be acceptable to some patients, having a stoma for a prolonged period is unlikely to appeal to most women, especially young women who might be in dating relationships.
While becoming pregnant may pose problems, most studies have shown that pregnancy following IPAA is safe and without an associated increase in maternal or fetal morbidity or mortality.113 Furthermore, there appears to be no increase in pouch-related complications or bowel obstruction during pregnancy. The concern with pregnancy is whether vaginal delivery should be recommended. Because of stool generally being semiformed in individuals with an IPAA, any degree of anal sphincter injury may lead to deterioration of functional results and, in particular, incontinence. For this reason many colorectal surgeons and obstetricians have recommended that women with an IPAA have a planned cesarean section. This is reflected by cesarean section rates of 38–78% after IPAA, which are considerably higher than the North American average of 22%.114 There are multiple retrospective studies, and no data to suggest that the risk of an anal sphincter tear is increased. Some women do experience transient worsening of their functional results during pregnancy, but there are no long-term differences in functional outcomes between patients who have a vaginal delivery compared with a cesarean section. Furthermore, there are data to suggest that women who have a pregnancy and vaginal delivery following IPAA have similar long-term function compared to those who did not have a pregnancy following IPAA. The difficulty with these data is that the series are small, and therefore the true rate of sphincter injury in this group is uncertain. The counter argument to planned cesarean section is that the morbidity to both the mother and fetus with cesarean section is generally higher than that with a vaginal delivery.
Complications Related to Colectomy and Ileorectal Anastomosis
The most significant complication following colectomy and ileorectal anastomosis (IRA) is an anastomotic leak. The reported rate is less than 5% indicating that patients who have a colectomy and IRA are highly selected.115,116 The presentation can vary from a small abscess treated with antibiotics and percutaneous drainage to a free leak within the abdominal cavity with peritonitis and overwhelming sepsis. The frequent use of steroids in this population can make the diagnosis of an anastomotic leak very difficult, with only subtle symptoms present at first, although rapid deterioration can occur and thus a high index of suspicion must be maintained at all times. In the event of a serious leak, reoperation with creation of an end ileostomy and mucous fistula is necessary.
Cancer in the Rectal Remnant
Patients who have had a colectomy and IRA continue to be at risk for cancer. Thus, surveillance of the rectum is required. The risk is increased if there was dysplasia or cancer in the resected colon, and this supports the view that colectomy and IRA is contraindicated in patients who have dysplasia or a cancer in the colon. The cumulative risk of cancer in a series of 374 patients who had been followed up to 23 years was 6% at 20 years and 15% at 30 years.49 Grundfest and colleagues reported a cumulative risk of cancer of 5% at 20 years and 12.9% at 25 years.117
Complications Related to the Kock Pouch
Valve malfunction, in particular valve slippage, has been the most frequent complication of the Kock pouch. In fact, it has been the Achilles heel of this procedure. Despite modifications in surgical technique, this complication occurs in 20–40% of individuals.65–67 Reoperation is required to repair the valve in virtually all patients.
Slippage of the valve refers to dessusception of the segment of bowel, used to create the nipple valve. Intussusception of the bowel is an abnormal physiologic state, and the bowel attempts to relieve it causing dessusception of the bowel and slippage of the valve. Detachment of the pouch from the abdominal wall likely precedes the actual dessusception. In fact, with modifications such as a mesenteric sling, it is unusual for the valve to completely dessuscept and more often the valve protrudes through the side of the pouch. This complication may occur any time after surgery but is most common in the first year after surgery. There are two characteristic manifestations: intubation of the pouch becomes difficult or impossible because of the angulation of the path of the bowel. In some instances, the patient may have to leave the catheter in continuously because reinsertion is impossible. The second symptom is incontinence, total or partial, depending on the degree of extrusion. The latter may be problematic because it is difficult for the patient to wear an appliance with the stoma being flush with the skin.
Patients suffering from valve slippage may present acutely because they are not been able to insert a catheter and empty the pouch. In these situations, sometimes a flexible scope can be used to intubate the pouch or, alternatively, an interventional radiologist may be able to insert a tube over a guide wire under fluoroscopy. As stated previously, surgery is required to definitively repair the valve. Most often, the valve can be reintussuscepted and fixed. Sometimes, however, the valve must be excised and another valve created from the afferent limb of the pouch.
Prolapse or procidentia of the valve is a less frequent complication. The valve remains intact but prolapses through the stoma. It is usually the result of an excessively large fascial opening. When pressure increases in the reservoir, there is no resistance to extrusion of the valve though the fascial opening. It can be prevented by creating a snug fascial opening. However, it may occur over time as the fascial opening enlarges. It may be corrected with a skin-level procedure in which the fascia is tightened by insertion of a few sutures or insertion of a piece of mesh. If this fails, the pouch may have to be resited at another site on the abdomen.
As with any gastrointestinal operation, fistulas may occur postoperatively due to inadvertant injury to the small bowel during the procedure, leaks from anastomotic suture lines, or erosion of intra-abdominal abscesses into the bowel. In addition, fistulas may arise from the nipple valve in this procedure. Most often, they arise from the fundus of the pouch at the base of the nipple valve or from the valve itself. Several factors make the fundus of the pouch a vulnerable site. First, there is often tension on this suture line because the valve is large and edematous when constructed while the reservoir has not yet dilated. Second, an anchoring suture placed between the fundus of the pouch and the abdominal wall might cut out, creating a perforation and fistula. Third, the bowel may be ischemic from passage of the stapler to maintain the valve. Finally, and probably most important, there may be erosion of the bowel in patients where a synthetic mesh has been used to stabilize the valve.67,71 In fact, when mesh is inserted, valve fistulas have been reported in approximately 25% of patients so many surgeons no longer use a mesh to stabilize the valve for this reason. Although the mesh decreases the risk of valve slippage, the risk of fistulization and septic complications is increased.67
Patients may present early or late. Often, they present first with a peristomal abscess or cellulitis. Subsequently there may be drainage of fecal material. Sometimes on scoping the patient, the mesh can be visualized at the base of the valve if it has eroded through. If a mesh has been used, the fistula will not close and surgery is required. This will require takedown of the pouch, excision of the valve, and creation of a new valve using the afferent limb of small bowel.
Cox and colleagues have reported the only case of adenocarcinoma involving a Kock pouch 28 years following construction.118 Hulten and colleagues examined and biopsied a cohort of 40 patients who had their Kock pouch for a mean duration of 30 years. There were no cases of high-grade dysplasia or cancer in any patients. This provides further evidence that cancer and dysplasia are rare events in the long term.119
Volvulus of the pouch is a rare complication but has been reported.118 Unfortunately, the diagnosis is usually made at laparotomy. In some instances, it may not be able to preserve the pouch.
It is likely that transient ischemia of the valve occurs frequently caused by the two to three rows of staples inserted to maintain the valve. Despite the valve and efferent conduit often appearing congested and dusky at the end of the procedure, it is unusual for this complication to occur. Ischemia and sloughing of the entire valve is a rare complication.
Complications Related to Ipaa
Leaks, Fistulas, and Septic Complications
The most important complication of IPAA surgery is an anastomotic leak. Leaks may occur either from the IAA or from the pouch itself. They may manifest as an asymptomatic leak found on contrast studies, a perianal, pelvic, or intra-abdominal abscess or a fistula. The latter may be communications from the pouch or IAA to other intra-abdominal structures, including the vagina or the abdominal or perianal skin. Most frequently, they occur within a few days of the procedure but may also occur many months after the procedure or closure of the ileostomy.120
Anastomotic leaks are significant not only because of their frequency but because they are the most common reason for pouch excision. In those in whom the pouch is not excised, functional results may be impaired. In our series of 1554 patients, septic complications occurred in 206 (13.3%) patients, and it was identified as the reason for pouch excision in 49 (46.2%) of all pouch excisions. IAA leaks accounted for 35% of septic complications while leaks from the pouch itself accounted for 19.4%.120 Gemlo and colleagues reported that perianal sepsis or pouch fistulas were the indication for pouch excision in 24% of their patients.121
The reported risk of an anastomotic leak is quite variable. Several factors may account for the variability. First, there is variability in reporting with some centers separating leaks, abscesses, and fistulas while others combining them. Second, this is a complication that has decreased significantly over time probably due to modifications in surgical technique as well as increasing experience with the procedure. Reported rates vary between 5 and 15%.120,122,123
Various patient factors may affect the leak rate, including disease activity and if the patient is on high doses of sterods. Higher leaks have also been reported in hand-sewn compared with stapled anastomosis. Ziv and colleagues analyzed 692 patients and found the rate of septic complications to be 10.5% in patients with hand-sewn anastomoses compared with 4.6% in those with stapled IPAA.81 In our series, the leak rate is 13.4% in patients having a hand-sewn anastomosis compared with 7.7% in those having a stapled anastomosis.82
A leak may manifest in various ways. One should have a high degree of suspicion that there may be a leak in individuals having a pouch without a covering ileostomy who develop a low-grade fever, pelvic or suprapubic pain, and/or an ileus. In these patients, a CT scan or pouchogram should be performed immediately. It is our experience that it is unusual for patients who do not have an ileostomy to develop generalized peritonitis and require an emergency operation. More often, they can be treated with antibiotics and prolonged drainage of the pouch. If there is an intra-abdominal or pelvic abscess, percutaneous drainage should be attempted. Even patients with a covering ileostomy may develop an intra-abdominal abscess that should be drained percutaneously. Sometimes a leak is not identified, but one must always be suspicious that there was one. One must also be cautious in closing the ileostomy of patients who had either a clinical or even a radiologic leak that appears to have healed on repeat pouchogram. Sometimes the leak may have sealed due to the pouch being defunctioned but has not healed fully, and the patient becomes symptomatic once the ileostomy is closed. In these individuals, an examination under anesthesia is warranted prior to ileostomy closure or sometimes a laparotomy depending on the degree of suspicion. However, despite these maneuvers, some patients will manifest with another leak or fistula following closure of the ileostomy.
While early on a leak most often led to excision of the pouch, now most pouches can be salvaged.124–126 Various modalities can be used, including antibiotics and drainage of the pouch (if there is no covering ileostomy), delayed closure of the ileostomy, and local techniques for repair of the anastomosis and reconstruction of the pouch with a combined abdominoperineal approach. It is our preference now is to undertake a combined abdominoperineal approach in most patients as the first procedure.127 It is often difficult to perform a local repair and advance the pouch in patients who had a stapled anastomosis previously. An advancement procedure is easier in patients who had a hand-sewn anastomosis, but there is more likely to be tension on the anastomosis. Also, with each attempt at repair, there is some degree of injury to the anal sphincter, and therefore, while a combined procedure is a major operation, it also may be more successful and lead to better long-term outcome. Reported rates of pouch salvage range from 70 to 80% of patients who suffer an anastomotic leak.
Pouch-vaginal fistula is a major complication following ileal pouch surgery. It is often more difficult to treat than other fistulas. The reported risk is in the range of 4–14%.128–130 It may develop early before ileostomy closure or more commonly a few months later. The vast majority occur at the ileoanal anastomotic level. It is likely that there are two mechanisms for their development. In women having a stapled IPAA, it is possible that the posterior wall of the vagina may be incorporated into the stapled anastomosis if care is not taken. Alternatively, and probably more commonly, a pouch-vaginal fistula is due to sepsis secondary to a leak at the IAA. Some women who develop a pouch-vaginal fistula are often diagnosed, in retrospect, to have Crohn's disease. In fact, if the fistula occurs a long time following surgery or there are other anal disease or pouch abnormalities, one should be suspicious of Crohn's disease.
Multiple treatment options have been described. The most common are local advancement of the pouch and combined abdominoperineal reconstruction of the pouch. Other methods include local repair, transvaginal repair, and interposition of a gracilis muscle. The choice of operation may depend on several factors. We have found local repairs to be more difficult to perform following stapled IPAA and prefer to perform a combined abdominoperineal operation especially if the fistula is at the anastomotic site. The pouch can be brought down beyond the fistula and the anastomosis performed at the dentate line. A local repair either performed transanally or transvaginally may be attempted in patients who had a hand-sewn anastomosis. Treatment in patients with suspected Crohn's disease may have to be dictated by the status of the pouch and whether there is other anal disease.
Unfortunately, the failure rate is higher than following repair of other IPAA fistulas, probably because of the scarring of the rectovaginal septum. Reported success rates are in the range of 60–70%.
Anal complications may occur early after the operation or many years later. The most common complication is anal stenosis. Rates of 11–38% have been reported.131,132 They occur more commonly after a hand-sewn anastomosis although many patients who have a stapled IPAA have a tight stricture while they are defunctioned with an ileostomy. However, in these patients, digital dilation at the time of closure of the ileostomy is usually adequate and recurrence is infrequent. Some stenoses are fibrostenotic in nature and likely occur secondary to a leak or sepsis. Tension on the anastomosis may also be a factor in their occurrence. Most strictures are mild and, because the stool is semiformed, they do not cause problems with evacuation. A small proportion may require dilation in the operating room. In the Mayo Clinic series, only one patient out of 1884 required excision of the pouch because of an anal stricture.133
In the long term, some patients may develop anal complications, especially fistulas and abscesses. One must always be suspicious that they have Crohn's disease. Abscesses should be drained. However, treatment of a fistula may be difficult. Fistulotomy should be discouraged because of the risk of incontinence even if the fistula is superficial. If the fistula is cryptoglandular in origin, it is usually not possible to perform an advancement procedure. Thus, if an abscess occurs infrequently, it may be prudent simply to treat symptomatically with antibiotics. For more symptomatic fistulas, a seton may be inserted. If the fistula is low, one might be able to use it as a cutting seton. Alternatively, one can allow the tract to epithelialize and then remove the seton. The patient may experience some minimal discharge from the fistula but not have recurrent abscesses, which is usually acceptable to most patients. Fibrin glue may be tried but has been unsuccessful in most patients in our experience.
Anal skin tags may be a problem for some patients. They may cause severe pain and irritation because of the stool frequency experienced by most patients with a pouch. As in other patients, excision of the tags should be avoided. However, if the tags are large and extremely symptomatic, they can be locally excised. If so, the patient should be warned of problems with nonhealing.
Because most surgeons prefer to perform the IPAA without performing a mucosectomy and instead perform a stapled anastomosis above the dentate line, a small segment of rectal mucosa remains. Ideally, only 1–2 cm of mucosa should be left behind. Most patients will have evidence of inflammation in this segment, but, despite this, most patients are asymptomatic or may complain of a small amount of blood within the stool.84 5-ASA or steroid suppositories can be prescribed, but often they are poorly tolerated. Rarely are the symptoms severe enough to require surgery, but, if so, a mucosectomy with advancement of the pouch can be performed or alternatively a combined reconstructive procedure with mucosectomy and hand-sewn anastomosis at the dentate line. Before embarking on treatment, the pouch should be scoped to confirm the diagnosis of cuffitis and rule out pouchitis.
Pouchitis is a nonspecific inflammation of the pouch mucosa, which is seen in patients with IPAA as well as those with a Kock pouch (Fig. 34-11).134 Clinically, pouchitis manifests with a variable spectrum of clinical symptoms, including increased stool frequency, rectal bleeding, abdominal cramping, rectal urgency and tenesmus, incontinence, and low-grade fever. On endoscopy, there are inflammatory changes that usually include mucosal edema, granularity, contact bleeding, loss of the vascular pattern, hemorrhage, and superficial ulceration. It is important that on histologic examination, there is evidence of acute inflammation including neutrophil infiltration.
Pouchitis. A. Endoscopic appearance. B. Radiologic appearance.
Patients often are labeled as having pouchitis when they have suboptimal function of the pouch. However, pouchitis is a specific diagnosis and should be based on clinical symptoms plus endoscopic and histologic changes. Svaninger and colleagues reported a cumulative risk of 34% in Kock pouch patients and 51% in IPAA patients at 5 years.135 However, approximately two-thirds had only one or a few episodes of pouchitis. Sandborn reported a risk of 15% at 1 year, 36% at 5 years, and 46% at 10 years.134 The etiology of pouchitis is unknown but is believed to be due to bacterial overgrowth. In addition, there may be an immune component because pouchitis rarely occurs in patients who have familial adenomatous polyposis. Other risk factors for pouchitis are anal strictures possibly leading to impaired pouch emptying. Also, patients with PSC appear to be at increased risk.136 High pANCA levels appear to be associated with the development of chronic pouchitis.137 In a series of 95 patients with ulcerative colitis who had IPAA, pouchitis developed in 42% of patients who were pANCA+ compared with 20% pANCA− patients. Similarly 56% of patients with high pANCA levels developed chronic pouchitis compared with only 20% who were pANCA−.
Antibiotics have been the mainstay of treatment for pouchitis. There is level I evidence that metronidazole and ciprofloxacin are effective in the treatment of pouchitis.138 Usually a 2-week course is instituted with response rates in the order of 75%. In most patients, the episode is short lived and rarely do patients develop recurrent episodes or chronic pouchitis. However, approximately 10–20% of patients may develop recurrent or chronic episodes of pouchitis. Probiotic therapy has been shown to decrease the risk of pouchitis and maintain a remission following an episode of pouchitis. Other agents including anti-inflammatory medications; steroids; immunosuppressive agents; free radical scavengers such as allopurinol, bismuth, and butyrate; and glutamine enemas have been tried with limited success.
Dysplasia and Malignancy Affecting the Pouch Mucosa.
Creation of the pouch results in stasis, creating a new ileal environment and mucosal adaptation of the pouch mucosa that may predispose to dysplasia. Lofberg et al published the first report of a patient who developed dysplasia and aneuploidy.139 Subsequently this group reported that 5 of 149 patients followed with serial biopsies were found to have dysplasia.140 The median time since construction of the pouch was 54 months (5–152 months). Four patients had low-grade dysplasia and one patient had sequential transformation into multifocal high-grade dysplasia. This group classified the histology of pouches as type A, B, or C. The five cases of dysplasia were found in the seven patients with persistent severe villous atrophy (type C histology).
Despite this finding, dysplasia appears to be a rare occurrence.141 There are only a few other reports in the literature. Thompson-Fawcett and colleagues examined a cohort of 116 patients considered to be at potentially high risk for developing dysplasia.142 Only one patient, a woman with a 23-year history of ulcerative colitis who had a pouch performed 14 years earlier, had low-grade dysplasia on one of the eight biopsies taken.
There are a few reports of cancer arising in the pouch, but, given the number of IPAA that have been performed in the past 30 years, it is not possible to conclude that the risk of cancer is in fact increased in patients with pouches because adenocarcinoma, although rare, does occur in the small bowel of normal individuals.141 However, it may be that there is a delay in the development of dysplasia, and with increasing time there may be more cases. Thus, it is difficult to make recommendations for follow-up at this time. It does appear that patients with chronic pouchitis and severe villous atrophy may be the group at highest risk and perhaps this is the group that should be followed with regular endoscopies and serial biopsies.
Dysplasia and Malignancy Affecting the Rectal Outlet.
Given that there is an increased risk of cancer in patients with ulcerative colitis, it is reasonable to expect that there may be an increased risk of cancer in the rectal outlet. Controversy exists as to whether a mucosectomy and hand-sewn anastomosis or a double-stapled anastomosis is preferable, especially in patients known to have cancer and dysplasia elsewhere. When this procedure was first described, it was assumed that by performing a mucosectomy that all mucosal cells would be extirpated. However, in O'Connell and colleagues' report of 29 patients who had excision of their pouches because of septic complications after mucosectomy and hand-sewn anastomosis83 14% had evidence of residual mucosa in the muscular cuff. They concluded that either mucosal cells remained following mucosectomy or there was regeneration of mucosa following mucosectomy. In either case, it is obvious that mucosectomy does not eliminate the risk of cancer.
There are nine reported cases of cancers involving the anal outlet.141 In three patients a mucosectomy had been performed while the rest had a stapled anastomosis. Of note is the fact that cancer or dysplasia was present in eight of the colectomy specimens. The data on dysplasia occurring in the anal outlet are less complete. O'Riordain and colleagues from the Cleveland Clinic have reported that dysplasia developed in the residual epithelial cuff in 7 of 210 patients who had had stapled ileal pouch anal anastomoses between 1987 and 1992.143 Dysplasia was high grade in one and low grade in six. Two patients had a mucosectomy performed while five were treated expectantly. In three of the seven patients, cancer or dysplasia had been present in the colectomy specimen suggesting that the dysplasia had been present at the time of surgery.
Thus, while patients with IPAA require follow-up, the method and frequency is not certain. It appears that the risk of cancer is low. Based on guidelines for surveillance of patients with ulcerative colitis who have not had surgery, a reasonable follow-up strategy would be to begin endoscopy and biopsy of the rectal outlet at 10 years and continue at 2-year intervals for individuals who have had a stapled anastomosis. For those who have had a mucosectomy and hand-sewn anastomosis, endoscopy and biopsy are not possible.
If one detects dysplasia, it is also difficult to know what to recommend. The biopsy specimens should definitely be reviewed by an experienced pathologist. Surgeons at the Cleveland Clinic have recommended performing a mucosectomy and advancement of the pouch with a hand-sewn IAA.143 However, this likely does not eliminate the risk of cancer given the experience with hand-sewn anastomosis and mucosectomy. Thus, excision of the pouch would be another option and would be our recommendation. However, this is a difficult decision, and certainly the alternatives should be discussed with the patient and the patient should participate in the decision making.
Mortality following surgery for ulcerative colitis is low. The contemporary mortality rate for subtotal colectomy in acute severe ulcerative colitis is 0–3%,56,144 but it increases dramatically when there is a colonic perforation. The mortality rate from large series of patients having IPAA is similarly low, in the range of 0–2%.145 This is probably because most patients undergoing IPAA are young and free of comorbid diseases.
On the other hand, morbidity rates are high following both urgent and elective procedures. Morbidity rates of 33–66% have been reported for patients having subtotal colectomy for acute colitis with the main complications being wound infection, ileus, small bowel obstruction, and a blown rectal stump.56,144 In patients having an IPAA, complication rates are nearly as high. In a Cochrane Review, complication rates (from all causes) were as high as 53%, including both procedure-specific and general complications following pouch construction.145 The rate of complications after laparoscopic IPAA were no different.
Long-Term Outcome Following Colectomy and Ileorectal Anastomosis
In a small series from the Mayo Clinic, 82% of patients had a functioning ileorectal anastomosis (IRA) at 5 years.116 The probability of having a functioning IRA in a series of 32 patients followed for an average of 3.5 years was 88%.146 Finally, a study from the Cleveland Clinic demonstrated that 54% of patients required excision of the rectum within 20 years of construction of an IRA, most commonly for ongoing symptoms, dysplasia, or malignancy.115 The same study, however, reported reasonable functional results, with patients having fewer bowel movements than matched controls who had an IPAA, but with more urgency.
Long-Term Outcome Following Kock Pouch
Revisional surgery is necessary in a large proportion of patients mainly because of valve complications. Kock reported a reoperative rate of 54% in his early series of patients, but the rate decreased to 16% subsequently.147 Despite the frequency of complications, most patients retain their pouch in the long term. Lepisto and Jarvinen reported a cumulative success rate of 96% at 1 year, 86% at 10 years, 77% at 15 years, and 71% at 29 years.65 In this series of 96 patients, 59% had required reconstructive surgery. Nessar et al reported 10- and 20-year pouch survival rates of 87–77%.66 In our own series of 194 patients, 81% of patients at 10 years and 67% at 20 years had a functioning Kock pouch.67 Wasmuth and Myrvold reported a failure rate of 11.6% at 20 years.68
Long-Term Outcome Following Ipaa
The reported failure rates are in the range of 5–10%. Many failures occur early, but there are an increased number of failures over time. In our own series of patients over 25 years, the failure rate was 6.8%.120 Median time to failure was 3.5 years. The risk factors for failure included Crohn's disease and a leak from the pouch or IAA on multivariable analysis. Gemlo et al reported a failure rate of 9.9% in 253 patients having surgery at the University of Minnesota.121 Poor functional results was the most common cause (28%), followed by unsuspected Crohn's disease (5%) and pelvic sepsis. Lepisto and Jarvinen reported an overall failure rate of 5.3%.65 The cumulative probability of pouch failure was 1% at 1 year, 5% at 5 years, and 7% at 10 years.
Experience with the procedure is also a factor in predicting success. In our own series, the complication, reoperative and failure rates dropped significantly over time. In the period 1981–84, the overall complication rate was 37.5%, the IAA leak rate was 30%, and the failure rate was 30%. There was a steady decrease so the respective rates in the period 1997–2000 were 10.6, 5.2, and 1.5%.148 Outcome was assessed in patients undergoing an IPAA over the period 1992–1998 using population data from the province of Ontario.149 Even though the surgical procedure had undergone significant modifications and been performed for more than 10 years by this time, a decrease in the complication rate as measured by readmission rate, reoperative rate, and failure rate was observed during that period. Also, outcome was significantly better in individuals having surgery in high-volume hospitals compared with medium- and low-volume hospitals.
Quality of Life and Functional Results
Ulcerative colitis has been shown to have a significant impact on quality of life, with disease activity being one of the largest predictors of outcome.150–152 In a population-based study from Norway, 328 patients with ulcerative colitis were evaluated with a Norwegian variation of the IBDQ (Inflammatory Bowel Disease Questionnaire). The frequency of disease relapse over a 5-year follow-up period was independently associated with a decrease in IBDQ scores.153 Thus, it is not surprising, given that surgery eliminates the disease, that most individuals who have had surgery for ulcerative colitis have a high quality of life. In fact, one of the first studies to document this was a study by Provenzale and colleagues who compared the outcome of 22 patients with IPAA to a normal population using the Short Form 36 (SF-36).61 They found that the quality of life of the individuals with IPAA was similar to that of the normal population. They also reported that the median utility for this cohort was 1.0 signifying normal health-related quality of life. Using the time trade-off technique, our group was able to show that the mean utility increased from 0.58 preoperatively to 0.98 at 1 year postoperatively in a cohort of 20 patients.62
As discussed previously in this chapter, surgeons have been innovative over the past 50 years in developing new procedures so that patients do not have to have a permanent ileostomy. In reality, most patients with conventional ileostomy have a high quality of life using the SF-36, time trade-off technique, and IBDQ; investigators have shown that not only is quality of life excellent following surgery but is similar irrespective of the procedure. Our group interviewed three cohorts of patients: 28 with conventional ileostomies, 28 with continent ileostomates, and 37 with pelvic pouches.62 The mean utilities, using the time trade-off technique were not significantly different with utilities ranging from 0.87 to 0.97 with a utility of one signifying perfect health. Jimmo and Hyman studied 12 patients who had a total proctocolectomy and ileostomy and 55 who had an IPAA. Overall, 46 of 55 (83.3%) patients with an IPAA and 10 of 12 (83.3%) with a proctocolectomy and ileostomy were satisfied with the procedure. All patients completed the IBDQ, and there was no significant difference between the two groups regarding the overall score or by category.63 Using a modified version of the SF-36, Thirlby and colleagues also showed that quality of life was equal or better than norms for the general population.64
This finding seems to contradict what occurs in clinical practice; that is, most patients, given the option, choose a pelvic pouch. Several reasons may explain the discrepancy. First, patients in these studies were not randomly allocated and they may choose their preferable option. Second, there may be an aspect of patients accepting their current health status and rationalizing that it is superior to other alternatives. Finally, the most important determinant of quality of life in these patients may be physical well-being that is improved in almost all patients after all procedures. What is important about these findings is that all of the various options should be presented to patients so they may choose which procedure is most acceptable to them and their lifestyle.
Recently, there have been several studies that have shown that quality of life is similar in patients having open or laparoscopic IPAA, but cosmesis and body image is better in patients having a laparoscopic IPAA.154
Following IPAA, the average number of bowel movements is approximately six per day. In a series of over 1300 patients followed for a median of 8 years, Farouk et al reported that 85% of patients were totally continent during the day but only 52% were totally continent at night. However, frequent incontinence was reported in less than 5% of patients.155 Several studies have shown that functional results deteriorate with advancing age. Also, functional results have been shown to correlate with quality of life.156 Kirat et al also reported better outcomes in patients having a stapled versus a hand-sewn IAA.157
Patients requiring surgery for ulcerative colitis have several options to choose from. A thorough understanding of the technical aspects of each procedure, their complications, and outcomes is essential in order to discuss the options with patients. Patients should be fully informed and they participate in the decision making. Although IPAA is the procedure of choice for most patients, they will have a good outcome with excellent quality of life irrespective of the procedure that is performed. However, to achieve such results, patients must be selected carefully and surgeons should be well versed in the technical details of the surgery as well as the pre- and postoperative care of patients and management of complications.